Background: Adenomyosis and congenital uterine anomalies (CUAs) can compromise reproductive potential and may coexist in the same patient, especially in cases of infertility. This review (CRD42022382850) aims to evaluate the published cases of concurrent adenomyosis and syndromic and nonsyndromic CUAs. Methods: A literature search for suitable articles published in the English language was performed using the following databases from inception to 30 November 2022: MEDLINE, EMBASE, Global Health, the Cochrane Library, Health Technology Assessment Database, and Web of Science. Articles including both CUAs and adenomyosis, with data about their potential relationship, were included. Results: The literature search retrieved 14 articles that met the purpose of this review and summarized the most recent findings regarding the concurrent diagnosis of adenomyosis and CUAs. Conclusions: Adenomyosis can be found in both syndromic and nonsyndromic CUAs, and may arise from several etiologies. The hypothesis that obstructions in CUAs increase uterine pressure and promote the development of adenomyosis remains to be further elucidated, and additional findings may also play a role. The patient’s genetic, epigenetic, and hormonal patterns, as well as normal physiological processes, such as pregnancy, may influence the growth of adenomyosis.